Citation and License

Arthritis Research & Therapy 2013, 15:R8
doi:10.1186/ar4136

Published: 9 January 2013

Abstract

Introduction

Microcirculation dysfunction is a typical feature of systemic sclerosis (SSc) and
represents the earliest abnormality of primary myocardial involvement. We assessed
coronary microcirculation status by combining two functional tests in SSc patients
and estimating its impact on disease outcome.

Methods

Forty-one SSc patients, asymptomatic for coronary artery disease, were tested for
coronary flow velocity reserve (CFR) by transthoracic-echo-Doppler with adenosine
infusion (A-TTE) and for left ventricular wall motion abnormalities (WMA) by dobutamine
stress echocardiography (DSE). Myocardial multi-detector computed tomography (MDCT)
enabled the presence of epicardial stenosis, which could interfere with the accuracy
of the tests, to be excluded. Patient survival rate was assessed over a 6.7- ± 3.5-year
follow-up.

Results

Nineteen out of 41 (46%) SSc patients had a reduced CFR (≤2.5) and in 16/41 (39%)
a WMA was observed during DSE. Furthermore, 13/41 (32%) patients showed pathological
CFR and WMA. An inverse correlation between wall motion score index (WMSI) during
DSE and CFR value (r = -0.57, P <0.0001) was observed; in addition, CFR was significantly reduced (2.21 ± 0.38) in
patients with WMA as compared to those without (2.94 ± 0.60) (P <0.0001). In 12 patients with abnormal DSE, MDCT was used to exclude macrovasculopathy.
During a 6.7- ± 3.5-year follow-up seven patients with abnormal coronary functional
tests died of disease-related causes, compared to only one patient with normal tests.

Conclusions

A-TTE and DSE tests are useful tools to detect non-invasively pre-clinical microcirculation
abnormalities in SSc patients; moreover, abnormal CFR and WMA might be related to
a worse disease outcome suggesting a prognostic value of these tests, similar to other
myocardial diseases.